不同类型儿童吉兰-巴雷综合征的临床特点及治疗随访研究

目的 探讨不同类型儿童吉兰-巴雷综合征(GBS)的临床特点及丙种球蛋白(IVIG)的治疗效果.方法 回顾性分析了我科近5年住院诊治的108例GBS患儿,其中本组75例患儿均在急性期应用大剂量IVIG 400 mg/(kg·d)静点治疗5 d,收集患儿的临床、电生理资料和治疗效果,并对患儿病情恢复进行随访.结果 75例GBS患儿中急性运动性轴索型GBS(AMAN)34例(45.3%),急性炎症性脱髓鞘多发性神经病(AIDP)32例(42.7%),急性运动感觉性轴索型GBS(AMSAN)3例(4.0%),神经失电位型4例(5.3%),难以分类2例(2.7%).AIDP型起病达病情高峰时间明显比AMAN型长,差异有统计学意义(t=3.4042,P＜0.01);病情高峰时Hughes功能障碍评分,AIDP和AMAN型差异无统计学意义(x2=1.5997,P＞0.05).二者在呼吸肌麻痹、颅神经麻痹及植物神经症状方面差异无统计学意义;AIDP型患儿感觉障碍症状明显多于AMAN型,二者差异有统计学意义(x2=6.0475,P＜0.05).经IVIG治疗后AIDP和AMAN型肌力开始改善平均时间分别为(5.59±3.63)、(7.21±4.68)d,二者经治疗肌力开始改善时间AIDP型较AMAN型短,但差异没有统计学意义(t=-1.5702,P＞0.05);肌力提高1级所需时间AIDP和AMAN型分别为(8.88±4.39)、(12.67±8.35)d,二者经治疗肌力提高一级的时间AIDP型比AMAN短4 d左右,差异有统计学意义(t=-2.3689,P＜0.05).本组无1例死亡,随访调查的病例中AIDP型和AMAN型治疗后完全恢复时间差异无统计学意义(t=0.2041,P＞0.05).结论 AMAN型患儿临床进展速度较AIDP型快,除感觉神经受累方面AIDP型多于AMAN型患儿外,二者在肌无力严重程度、呼吸肌麻痹、颅神经麻痹及植物神经受累方面无明显差异.经IVIG治疗AIDP型临床恢复比AMAN型快,但AIDP和AMAN型长期预后无明显差异.

Abstract：

Objective To study the clinical characteristics and effects of immunoglobulin treatment hospitalized for GBS were retrospectively analyzed; 75 cases in this group were given acute high dose of gamma globulin(IVIG)400mg/(kg·d)intravenously for 5d.Clinical and electrophysiological data and information on treatment and recovery of the children were collected during the follow-up and were analyzed.Result According to the clinical and electrophysiologic findings, 32 patients manifested acute inflammatory demyelinating polyradiculoneuropathy( AIDP), 34 had acute motor axonal neuropathy( AMAN), 3 had acute motor and sensory axonal neuropathy (AMSAN), 4 were inexcitable, 2 were unclassified. The clinical progress of the AMAN was faster than the AIDP group. Except for sensory nerve involvement, there was no significant difference in the clinical feature and severity. The mean time of the muscle strength began to recover was (5.59 +3.63) days in the AIDP group and (7. 21 ±4.68) days in the AMAN group after IVIG treatment. The time of the AIDP group was shorter than the AMAN group, but the difference was not statistically significant ( t = - 1. 5702, P ＞ 0. 05 ). The mean time of the muscle strength increased one grade was (8.88 ±4. 39) days in the AIDP group and ( 12. 67 ±8. 35) days in the AMAN group. The difference was statistically significant ( t = - 2. 3689, P ＜ 0. 05 ). No patients in this group died. Follow-up data showed that the complete recovery time was not significantly different ( t = 0. 2041, P ＞ 0. 05 ). Conclusion The clinical progress of the AMAN was faster than the AIDP group. Besides sensory nerve involvement,there was no significant difference in the clinical feature and severity. The AIDP group's clinical recovery was faster than AMAN's after the immunoglobulin treatment. The two groups were not significantly different in long-term prognosis.     

运动神经传导阻滞与儿童吉兰-巴雷综合征不同亚型间的关系

目的 探讨运动神经传导阻滞（CB）与儿童吉兰-巴雷综合征（GBS）不同亚型间的关系。方法 回顾性分析50例GBS患儿的临床资料和神经电生理资料,根据神经电生理结果分为2型：急性炎症性脱髓鞘性多发性神经根神经病（AIDP,n=29）和急性运动轴索型神经病（AMAN,n=21）。根据有无运动神经CB分为伴有运动神经CB的AMAN（n=10）、不伴有运动神经CB的AMAN（n=11）、伴有运动神经CB的AIDP（n=19）和不伴有运动神经CB的AIDP（n=10）。比较各组间患儿起病年龄、性别、疾病高峰期休斯功能分级量表（HFGS）评分、短期预后（起病1个月后HFGS评分）。结果 AMAN中,运动神经CB均为可逆性。伴有运动神经CB的AMAN的起病1个月后HFGS评分低于不伴有运动神经CB的AMAN（P < 0.05）,伴有运动神经CB的AIDP的起病1个月后HFGS评分高于伴有运动神经CB的AMAN（P < 0.05）。结论 伴有可逆性运动神经CB的AMAN提示神经纤维病变轻微,短期预后较不伴有运动神经CB的AMAN和AIDP恢复快。     

Clinical presentation and prognosis of childhood Guillain-Barré syndrome

Aim:   Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterised by rapidly progressive, symmetric weakness and areflexia. This study is to assess the clinical characteristics of paediatric GBS, as well as its long-term functional prognosis.
Methods:   We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 56 children diagnosed with GBS. Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy ([AIDP] n  = 34), acute motor axonal neuropathy ([AMAN] n  = 14), acute motor and sensory axonal neuropathy ( n  = 1) and Miller Fisher syndrome ([MFS] n  = 7).
Results:   Upper respiratory infection was the most frequent preceding event, and limb weakness was the most frequent symptom at GBS onset. There was no significant difference in the mean time from the onset of illness to nadir between any of these groups. Both the AIDP and AMAN groups showed significantly poorer functional status, measured by the Hughes scale, than the MFS group. Two years after nadir, however, the three groups did not differ significantly. Functional status at nadir, as estimated by the Hughes scale, is a more important factor than electrophysiological types in predicting long-term outcome.
Conclusion:   The most common symptom at onset in paediatric GBS was limb weakness. Functional status at nadir in AMAN was not significantly different from that of AIDP, and both types achieved good functional outcome for ambulation after 2 years. Functional status at nadir was more important than the electrophysiological type in predicting long-term outcomes.     

运动神经传导阻滞与儿童吉兰-巴雷综合征不同亚型间的关系

目的 探讨运动神经传导阻滞（CB）与儿童吉兰-巴雷综合征（GBS）不同亚型间的关系。方法 回顾性分析50例GBS患儿的临床资料和神经电生理资料,根据神经电生理结果分为2型：急性炎症性脱髓鞘性多发性神经根神经病（AIDP,n=29）和急性运动轴索型神经病（AMAN,n=21）。根据有无运动神经CB分为伴有运动神经CB的AMAN（n=10）、不伴有运动神经CB的AMAN（n=11）、伴有运动神经CB的AIDP（n=19）和不伴有运动神经CB的AIDP（n=10）。比较各组间患儿起病年龄、性别、疾病高峰期休斯功能分级量表（HFGS）评分、短期预后（起病1个月后HFGS评分）。结果 AMAN中,运动神经CB均为可逆性。伴有运动神经CB的AMAN的起病1个月后HFGS评分低于不伴有运动神经CB的AMAN（P < 0.05）,伴有运动神经CB的AIDP的起病1个月后HFGS评分高于伴有运动神经CB的AMAN（P < 0.05）。结论 伴有可逆性运动神经CB的AMAN提示神经纤维病变轻微,短期预后较不伴有运动神经CB的AMAN和AIDP恢复快。     

儿童急性运动轴索性多发性神经病临床-电生理特征及预后

目的：探讨中国南方儿童急性运动轴索性多发性神经病（acute motor axonal neuropathy, AMAN）的临床特征、电生理特点及其预后。方法：对6例AMAN患儿的临床和神经电生理资料进行分析和随访。结果：（1）患儿平均发病年龄为4.4岁,均来自农村,多有前驱感染史（5例）,发病时间无明显季节性差异。肌无力为主要首发症状,病情达高峰的平均时间为4.2 d。（2）神经电生理检查：所有患儿神经传导检测均表现为运动神经反应电位波幅显著减低,降低至（22.3%～73.4%）正常值低限,四肢远端神经受损程度较近端重（P0.05）,运动神经电位传导速度和感觉神经传导正常。全部患儿给予静脉丙种球蛋白（IVIG）治疗。（3）随访3个月至1年,4例患儿已能独立行走。结论：儿童AMAN好发于农村,全年均可发病;最常见的首发症状为肌无力,多在病程早期达到疾病高峰;神经电生理对AMAN的诊断、鉴别诊断提供了客观依据;部分患儿起病后1年能恢复独立行走能力;尽早IVIG治疗有利于促进临床神经功能恢复。     

急性感染性多发性神经根炎患儿空肠弯曲菌感染与抗神经节苷脂GM_1抗体关系

目的：急性感染性多发性神经根炎(GBS)病因尚不清楚,目前认为与感染,尤其与空肠弯曲菌(CJ)感染有关,本文研究CJ感染与神经节苷脂(GM1)损伤的关系,探讨GBS的发病机理。方法：采用酶联免疫吸附试验测定31例GBS患儿(经典型急性感染性多发性神经根炎AIDP 23例,急性运动性轴素神经病AMAN 8例)急性期、恢复期血清和急性期脑脊液CJ-IgG抗体及GM1-IgG、GM1-IgM抗体的变化;并与非GBS神经系统疾病患儿(NGBS组)和10例正常儿童(正常组)对比。结果：AMAN急性期、恢复期血清CJ-IgG抗体水平高于NGBS组(P0.05)。GBS组脑脊液GM1-IgM水平高于NGBS组(P<0.05)。CJ-IgG与GM1-IgG、GM1-IgM具有明显的相关性(R=0.722,P=0.05)。结论：空肠弯曲菌感染是GBS发病的重要病因。神经节苷脂GM1的免疫损伤在GBS发病中起重要作用。     

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??Objective??To explore the clinical features of acute poisoning in children to search for effective preventive measures. Methods??A total of 521 cases of acute poisoning in Xi’an Children’s Hospital from January 2012 to January 2017 were collected and all clinical data were retrospectively analyzed. Results??Among the 521 cases of acute poisoning??291 cases??55.9%?? were boys??and 230 were girls??44.1%??. Acute poisoning occured in all ages of children and children in infancy period and pre-school period were more susceptible??257 cases and 147 cases???? poisoning occured mostly at home??508 cases?? accounting for 97.5%???? and in the countryside??431cases?? 82.7%???? the most common season was summer??187cases??35.9%??. The intoxication from digestive tract was the most common in all intoxicated pathways ??492 cases??94.4%??. After timely and reasonable treatment??most children had a good prognosis. Conclusion??Acute poisoning is most common in infants and preschool children. The majority of the patients are from rural areas. The most common season is summer. The most common intoxication is digestive tract.     

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??Abstracts?? Objective To summarize the clinical characteristics of infantile-onset autoimmune myasthenia gravis and improve diagnostic skills. Methods The investigation summarized retrospectively the features including clinical manifestation?? laboratory examination and the prognosis of infantile-onset autoimmune myasthenia gravis from June 2006 to February 2012 in 16 patients in Neurology Department of Beijing Children’s Hospital affiliated to Capital University of Medicine??and analysed the difference of the clinical classification and myasthenia crisis incidence between infatile group and the more than 1 year group during the corresponding period. Results Sixteen cases were revolved?? including 9 boys and 7 girls. The youngest was 4 months old?? the average age was 7.6 months. The average course of disease was 1.8 months at first visiting. Main symptoms included ptosis?? poor spirit and dysphagia.Among them 4 cases were misdiagnosed as central nervous system infection and 1 as digestive system disease . Ocular and generalized type was respectively 4??25%?? and 12??75%?? cases?? 4 ??25%?? cases underwent myasthenia crisis. Generalized myasthenia gravis component ratio and myasthenia crisis incidence rate of infantile-onset autoimmune myasthenia gravis was increasing significantly. Positive rate of Neostigmine test?? acetylcholine receptor antibody and repetitive nerve stimulation was respectively 93.7%??57% and 50%??and no significance was found between ocular and generalized type. All 16 cases received pyridostigmine bromide therapy?? 15 cases received glucocorticoids therapy. Mean improvement time was??6.8±5.6?? days after glucocorticoids therapy??but 1 of them deteriorate 6 days later??10 chlidren was follwed-up for 1 year and 1 month to 5 years and 3 months??3 cases were with completely stable remission??CSR????1 case pharmacologic remission??PR????4 cases were minimal manifestation??MM????1 case was improved??I?? and 1 case was unchanged??U??. Conclusion Infantile-onset autoimmune myasthenia gravis ??mainly manifested as generalized type??cam be easily misdiagnosed because of atypical onset-symptoms?? and incidence rate of myasthenia crisis is high.     

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目的探讨连续性静-静脉血液透析/滤过(CVVHDF)对小儿急性肝功能衰竭的治疗作用。方法 2009年5月至2010年5月期间,采用CVVHDF辅助治疗上海交通大学附属儿童医院重症监护病房(PICU)收治的急性肝功能衰竭患儿共5例。其中男2例,女3例。年龄10个月至5岁。入院时发病时间3～14d。药物性肝损(对乙酰氨基酚)3例,严重脓毒症合并肝损1例,阑尾炎术后门静脉炎引起的肝损1例。肝性脑病分级:2级1例,3级2例,4级2例。观察治疗前后病情及肝功能指标,并观察处理并发症。结果 CVVHDF治疗开始时间为入院后(2～24h),治疗时间为(24～144h),5例患儿中治愈3例,自动出院1例,死亡1例。CVVHDF治疗后12h,丙氨酸转氨酶(ALT)从(3888.76±2373.60)U/L下降至(3284.80±1974.80)U/L,治疗后24～48h继续下降,差异有统计学意义(F=3.58,P<0.05);CVVHDF治疗后12h,血氨从(209.00±53.61)μmol/L下降至(158.80±60.93)μmol/L,差异有统计学意义,以后继续好转(F=3.75,P<0.05)。CVVHDF治疗后12...